Page last updated: 2024-10-06

Prolinemia Type II

Proteins (20)

ProteinSynonymsTaxonomy
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrialP5C dehydrogenase; EC 1.2.1.88; Aldehyde dehydrogenase family 4 member A1; L-glutamate gamma-semialdehyde dehydrogenaseHomo sapiens (human)
D-amino-acid oxidaseDAAO; DAMOX; DAO; EC 1.4.3.3Homo sapiens (human)
Nitric oxide synthase, brainEC 1.14.13.39; Constitutive NOS; NC-NOS; NOS type I; Neuronal NOS; N-NOS; nNOS; Peptidyl-cysteine S-nitrosylase NOS1; bNOSHomo sapiens (human)
Pyrroline-5-carboxylate reductase 2P5C reductase 2; P5CR 2; EC 1.5.1.2Homo sapiens (human)
Ornithine aminotransferase, mitochondrialEC 2.6.1.13; Ornithine delta-aminotransferase; Ornithine--oxo-acid aminotransferaseHomo sapiens (human)
Creatine kinase B-typeEC 2.7.3.2; Brain creatine kinase; B-CK; Creatine kinase B chain; Creatine phosphokinase B-type; CPK-BHomo sapiens (human)
Aspartate aminotransferase, cytoplasmiccAspAT; EC 2.6.1.1; EC 2.6.1.3; Cysteine aminotransferase, cytoplasmic; Cysteine transaminase, cytoplasmic; cCAT; Glutamate oxaloacetate transaminase 1; Transaminase AHomo sapiens (human)
Carbamoyl-phosphate synthase [ammonia], mitochondrialEC 6.3.4.16; Carbamoyl-phosphate synthetase I; CPSase IHomo sapiens (human)
Guanidinoacetate N-methyltransferaseEC 2.1.1.2Homo sapiens (human)
Arginase-1EC 3.5.3.1; Liver-type arginase; Type I arginaseHomo sapiens (human)
Ornithine transcarbamylase, mitochondrialOTCase; EC 2.1.3.3; Ornithine carbamoyltransferase, mitochondrialHomo sapiens (human)
Bifunctional glutamate/proline--tRNA ligaseBifunctional aminoacyl-tRNA synthetase; Cell proliferation-inducing gene 32 protein; Glutamatyl-prolyl-tRNA synthetaseHomo sapiens (human)
Argininosuccinate synthaseEC 6.3.4.5; Citrulline--aspartate ligaseHomo sapiens (human)
Glutamate dehydrogenase 1, mitochondrialGDH 1; EC 1.4.1.3Homo sapiens (human)
Prolyl 4-hydroxylase subunit alpha-34-PH alpha-3; EC 1.14.11.2; Procollagen-proline,2-oxoglutarate-4-dioxygenase subunit alpha-3Homo sapiens (human)
Proline dehydrogenase 1, mitochondrialEC 1.5.5.2; Proline oxidase; Proline oxidase 2; p53-induced gene 6 proteinHomo sapiens (human)
Argininosuccinate lyaseASAL; EC 4.3.2.1; ArginosuccinaseHomo sapiens (human)
Glycine amidinotransferase, mitochondrialEC 2.1.4.1; L-arginine:glycine amidinotransferase; TransamidinaseHomo sapiens (human)
Probable arginine--tRNA ligase, mitochondrialEC 6.1.1.19; Arginyl-tRNA synthetase; ArgRSHomo sapiens (human)
Mitochondrial ornithine transporter 1Solute carrier family 25 member 15Homo sapiens (human)

Compounds (33)

CompoundDescription
phosphoric acidconcise etchant is 37% H3PO4
hydronium ion
Orthophosphate
succinic acidA water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851)
ureaA compound formed in the liver from ammonia produced by the deamination of amino acids. It is the principal end product of protein catabolism and constitutes about one half of the total urinary solids.
Nitric OxideA free radical gas produced endogenously by a variety of mammalian cells, synthesized from ARGININE by NITRIC OXIDE SYNTHASE. Nitric oxide is one of the ENDOTHELIUM-DEPENDENT RELAXING FACTORS released by the vascular endothelium and mediates VASODILATION. It also inhibits platelet aggregation, induces disaggregation of aggregated platelets, and inhibits platelet adhesion to the vascular endothelium. Nitric oxide activates cytosolic GUANYLATE CYCLASE and thus elevates intracellular levels of CYCLIC GMP.
ammonium hydroxideThe hydroxy salt of ammonium ion. It is formed when AMMONIA reacts with water molecules in solution.
Carbon DioxideA colorless, odorless gas that can be formed by the body and is necessary for the respiration cycle of plants and animals.
glutamic acidA non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.
NADH
creatineAn amino acid that occurs in vertebrate tissues and in urine. In muscle tissue, creatine generally occurs as phosphocreatine. Creatine is excreted as CREATININE in the urine.
Adenosine TriphosphateAn adenine nucleotide containing three phosphate groups esterified to the sugar moiety. In addition to its crucial roles in metabolism adenosine triphosphate is a neurotransmitter.
adenosine diphosphateAdenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.
adenosine monophosphateAdenine nucleotide containing one phosphate group esterified to the sugar moiety in the 2'-, 3'-, or 5'-position.
ornithineAn amino acid produced in the urea cycle by the splitting off of urea from arginine.
Hydrogen PeroxideA strong oxidizing agent used in aqueous solution as a ripening agent, bleach, and topical anti-infective. It is relatively unstable and solutions deteriorate over time unless stabilized by the addition of acetanilide or similar organic materials.
WaterA clear, odorless, tasteless liquid that is essential for most animal and plant life and is an excellent solvent for many substances. The chemical formula is hydrogen oxide (H2O). (McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed)
OxygenAn element with atomic symbol O, atomic number 8, and atomic weight [15.99903; 15.99977]. It is the most abundant element on earth and essential for respiration.
3-hydroxy delta 1-pyrroline-5-carboxylic acidRN given refers to 3,4-dihydro-4-hydroxy-2H-pyrrole-2-carboxylic acid
delta-1-pyrroline-5-carboxylate(L)-isomer is the biologically active form; RN given refers to cpd without isomeric designation; structure
prolineA non-essential amino acid that is synthesized from GLUTAMIC ACID. It is an essential component of COLLAGEN and is important for proper functioning of joints and tendons.
Argininosuccinic AcidThis amino acid is formed during the urea cycle from citrulline, aspartate and ATP. This reaction is catalyzed by argininosuccinic acid synthetase.
glutamic acid gamma-semialdehydestraight-chain form of delta(1)-pyrroline-5-carboxylate; RN given refers to cpd with unspecified isomeric designation
carbamyl phosphateThe monoanhydride of carbamic acid with PHOSPHORIC ACID. It is an important intermediate metabolite and is synthesized enzymatically by CARBAMYL-PHOSPHATE SYNTHASE (AMMONIA) and CARBAMOYL-PHOSPHATE SYNTHASE (GLUTAMINE-HYDROLYZING).
3,4-dihydro-2h-pyrrole-5-carboxylic acidstructure
alpha-ketoglutaric acid
NADA coenzyme composed of ribosylnicotinamide 5'-diphosphate coupled to adenosine 5'-phosphate by pyrophosphate linkage. It is found widely in nature and is involved in numerous enzymatic reactions in which it serves as an electron carrier by being alternately oxidized (NAD+) and reduced (NADH). (Dorland, 27th ed)
2-keto-4-hydroxyglutarateRN given refers to parent cpd
oxaloacetic acidA dicarboxylic acid ketone that is an important metabolic intermediate of the CITRIC ACID CYCLE. It can be converted to ASPARTIC ACID by ASPARTATE TRANSAMINASE.
s-adenosylmethioninePhysiologic methyl radical donor involved in enzymatic transmethylation reactions and present in all living organisms. It possesses anti-inflammatory activity and has been used in treatment of chronic liver disease. (From Merck, 11th ed)
hydroxyprolineA hydroxylated form of the imino acid proline. A deficiency in ASCORBIC ACID can result in impaired hydroxyproline formation.
creolinfrom refined coal tar oils
pyrophosphate